Frontotemporal dementia

Overview

Frontotemporal dementia (FTD) is an umbrella term for a group of brain diseases that mainly affect the frontal and temporal lobes of the brain. These areas of the brain are associated with personality, behavior and language.

In frontotemporal dementia, parts of these lobes shrink, known as atrophy. Symptoms depend on which part of the brain is affected. Some people with frontotemporal dementia have changes in their personalities. They become socially inappropriate and may be impulsive or emotionally indifferent. Others lose the ability to properly use language.

Frontotemporal dementia can be misdiagnosed as a mental health condition or as Alzheimer's disease. But FTD tends to occur at a younger age than does Alzheimer's disease. It often begins between the ages of 40 and 65, although it can occur later in life as well. FTD is the cause of dementia about 10% to 20% of the time.

Symptoms

Symptoms of frontotemporal dementia differ from one person to the next. Symptoms get worse over time, usually over years.

People with frontotemporal dementia tend to have clusters of symptom types that occur together. They also may have more than one cluster of symptom types.

Behavioral changes

The most common symptoms of frontotemporal dementia involve extreme changes in behavior and personality. These include:

• Increasingly inappropriate social behavior.
• Loss of empathy and other interpersonal skills. For example, not being sensitive to another person's feelings.
• Lack of judgment.
• Loss of inhibition.
• Lack of interest, also known as apathy. Apathy can be mistaken for depression.
• Compulsive behaviors such as tapping, clapping, or smacking lips over and over.
• A decline in personal hygiene.
• Changes in eating habits. People with FTD typically overeat or prefer to eat sweets and carbohydrates.
• Eating objects.
• Compulsively wanting to put things in the mouth.

Speech and language symptoms

Some subtypes of frontotemporal dementia lead to changes in language ability or loss of speech. Subtypes include primary progressive aphasia, semantic dementia and progressive agrammatic aphasia, also known as progressive nonfluent aphasia.

These conditions can cause:

• Increasing trouble using and understanding written and spoken language. People with FTD may not be able to find the right word to use in speech.
• Trouble naming things. People with FTD may replace a specific word with a more general word, such as using "it" for pen.
• No longer knowing word meanings.
• Having hesitant speech that may sound telegraphic by using simple, two-word sentences.
• Making mistakes in sentence building.

Movement conditions

Rare subtypes of frontotemporal dementia cause movements similar to those seen in Parkinson's disease or amyotrophic lateral sclerosis (ALS).

Movement symptoms may include:

• Tremor.
• Rigidity.
• Muscle spasms or twitches.
• Poor coordination.
• Trouble swallowing.
• Muscle weakness.
• Inappropriate laughing or crying.
• Falls or trouble walking.

Causes

In frontotemporal dementia, the frontal and temporal lobes of the brain shrink and certain substances build up in the brain. What causes these changes is usually not known.

Some genetic changes have been linked to frontotemporal dementia. But more than half of the people with FTD have no family history of dementia.

Researchers have confirmed that some frontotemporal dementia gene changes also are seen in amyotrophic lateral sclerosis (ALS). More research is being done to understand the connection between the conditions.

Risk factors

Your risk of getting frontotemporal dementia is higher if you have a family history of dementia. There are no other known risk factors.

Diagnosis

There's no single test for frontotemporal dementia. Healthcare professionals consider your symptoms and exclude other possible causes of your symptoms. FTD can be hard to diagnose early because symptoms of frontotemporal dementia often overlap with those of other conditions. Healthcare professionals may order the following tests.

Blood tests

To help rule out other conditions, such as liver or kidney disease, you may need blood tests.

Sleep study

Some symptoms of obstructive sleep apnea can be similar to those of frontotemporal dementia. These symptoms can include changes in memory, thinking and behavior. You may need a sleep study if you experience loud snoring and pauses in breathing while you sleep. A sleep study can help rule out obstructive sleep apnea as a cause of your symptoms.

Neuropsychological testing

Healthcare professionals may test your reasoning and memory skills. This type of testing is especially helpful to learn which type of dementia you may have at an early stage. It also can help distinguish FTD from other causes of dementia.

Brain scans

Images of the brain can reveal visible conditions that may be causing symptoms. These may include clots, bleeding or tumors.

• Magnetic resonance imaging (MRI). An MRI machine uses radio waves and a strong magnetic field to produce detailed images of the brain. An MRI can show changes in the shape or size of the frontal or temporal lobes.
• Fluorodeoxyglucose positron emission tracer (FDG-PET) scan. This test uses a low-level radioactive tracer that's injected into the blood. The tracer can help show areas of the brain where nutrients are poorly metabolized. Areas of low metabolism can show where changes have occurred in the brain and can help doctors diagnose the type of dementia.

There's hope that diagnosing frontotemporal dementia may become easier in the future. Researchers are studying potential biomarkers of FTD. Biomarkers are substances that can be measured to help diagnose a disease.

Treatment

There's currently no cure or treatment for frontotemporal dementia, although research into treatments is ongoing. Medicines used to treat or slow Alzheimer's disease don't seem to be helpful for people with frontotemporal dementia. Some Alzheimer's medicines may worsen the FTD symptoms. But certain medicines and speech therapy can help manage your symptoms.

Medications

These medicines may help manage behavioral symptoms of frontotemporal dementia.

• Antidepressants. Some types of antidepressants, such as trazodone, may reduce behavioral symptoms. Selective serotonin reuptake inhibitors (SSRIs) also are effective for some people. They include citalopram (Celexa), escitalopram (Lexapro), paroxetine (Paxil, Brisdelle) or sertraline (Zoloft).
• Antipsychotics. Antipsychotic medicines, such as olanzapine (Zyprexa) or quetiapine (Seroquel), are sometimes used to treat behavioral symptoms of FTD. But these medicines must be used with caution in people with dementia. They can have serious side effects, including an increased risk of death.

Therapy

People with frontotemporal dementia who have trouble with language may benefit from speech therapy. Speech therapy teaches people to use communication aids.

Lifestyle and home remedies

As frontotemporal dementia symptoms get worse, you'll need the help of caregivers. Caregivers can help you with daily activities, help maintain your safety, provide transportation and help with finances. Your healthcare professional can tell you when you may need to stop driving or let someone you trust take over your finances.

It's also important to get regular cardiovascular exercise. It may help improve your mood and thinking skills.

At home, it may be helpful to make adjustments so that daily living tasks are easier and you reduce the chance of injuries. For example, remove rugs to reduce falls. Or raise toilets to make it easier to use the bathroom.

Caregivers may be able to address behavioral symptoms by changing the way they interact with people with dementia. Ask your loved one's healthcare professional about resources that provide training in caring for someone with dementia.

Keeping a log of behavioral symptoms may help you pinpoint things in the environment that trigger symptoms. Taking these steps also may help:

• Skip events or activities that trigger behavior that's not desired.
• Remove negative environmental cues, such as car keys.
• Maintain a calm environment.
• Provide structured routines.
• Simplify daily tasks.
• Distract and redirect attention from harmful or inappropriate behaviors.

Coping and support

If you've been diagnosed with frontotemporal dementia, receiving support, care and compassion from people you trust can be invaluable.

Through your healthcare professional or the internet, find a support group for people with frontotemporal dementia. A support group can provide information tailored for your needs. It also allows you to share your experiences and feelings.

For caregivers and care partners

Caring for someone with frontotemporal dementia can be challenging because FTD can cause extreme personality changes and behavioral symptoms. It may be helpful to educate others about behavioral symptoms and what they can expect when spending time with your loved one.

Caregivers and the spouses, partners or other relatives who care for people with dementia, known as care partners, need assistance. They might find help from family members, friends and support groups. Or they may use respite care provided by adult care centers or home health care agencies.

It's important for caregivers and care partners to take care of their health, exercise, eat a healthy diet and manage their stress. Participating in hobbies outside the home may help ease some stress.

When a person with frontotemporal dementia requires 24-hour care, most families turn to nursing homes. Plans made ahead of time will make this transition easier and may allow the person to be involved in the decision-making process.

Preparing for an appointment

People with frontotemporal dementia often don't recognize that they have symptoms. Family members usually notice changes and arrange for an appointment with a healthcare professional.

Your healthcare professional may refer you to a doctor trained in nervous system conditions, known as a neurologist. Or you may be referred to a doctor trained in mental health conditions, known as a psychologist.

What you can do

You may not be aware of all your symptoms, so it's a good idea to take a family member or close friend along with you to your appointment. You also may want to take a written list that includes:

• Detailed descriptions of your symptoms.
• Medical conditions you've had in the past.
• The medical conditions of your parents or siblings.
• All the medicines and dietary supplements you take.
• Questions you want to ask your healthcare professional.

What to expect from your doctor

In addition to a physical exam, your healthcare professional checks your neurological health. This is done by testing things such as your balance, muscle tone and strength. You also may have a brief mental status evaluation to check your memory and thinking skills.